{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,26]],"date-time":"2025-10-26T21:16:50Z","timestamp":1761513410220},"reference-count":15,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2014,1,27]],"date-time":"2014-01-27T00:00:00Z","timestamp":1390780800000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J Med Case Reports"],"published-print":{"date-parts":[[2014,12]]},"abstract":"<jats:title>Abstract<\/jats:title>\n          <jats:sec>\n            <jats:title>Introduction<\/jats:title>\n            <jats:p>Dermatomyositis is a chronic inflammatory disorder characterized by muscular and dermatologic symptoms with variable internal organ involvement. This is the first report on a patient with acute dermatomyositis and fulminant systemic capillary leak syndrome.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Case presentation<\/jats:title>\n            <jats:p>A 69-year-old Caucasian woman with chronic dermatomyositis presented with clinical signs of severe hypovolemic shock and pronounced hemoconcentration (hematocrit, 69%). Her colloid osmotic pressure was 4.6mmHg. Following a bolus dose of prednisolone (500mg), fluid resuscitation was initiated. During volume loading, anasarca and acute respiratory distress rapidly developed. Echocardiography revealed an underfilled, hypokinetic, diastolic dysfunctional left ventricle with pericardial effusion but no signs of tamponade. Despite continued fluid resuscitation and high-dosed catecholamine therapy, the patient died from refractory shock 12\u00a0hours after intensive care unit admission. A laboratory analysis of her complement system suggested the presence of C1 inhibitor deficiency as the cause for systemic capillary leakage. The post-mortem examination revealed bilateral pleural, pericardial and peritoneal effusions as well as left ventricular hypertrophy with patchy myocardial fibrosis. Different patterns of endomysial\/perimysial lymphocytic infiltrations adjacent to degenerated cardiomyocytes in her myocardium and necrotic muscle fibers in her right psoas major muscle were found in the histological examination.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Conclusions<\/jats:title>\n            <jats:p>This case report indicates that acute exacerbation of chronic dermatomyositis can result in a fulminant systemic capillary leak syndrome with intense hemoconcentration, hypovolemic shock and acute heart failure. In the presented patient, the cause for diffuse capillary leakage was most probably acquired angioedema, a condition that has been associated with both lymphoproliferative and autoimmunologic disorders.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1186\/1752-1947-8-28","type":"journal-article","created":{"date-parts":[[2014,1,27]],"date-time":"2014-01-27T18:02:38Z","timestamp":1390845758000},"update-policy":"http:\/\/dx.doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Fulminant systemic capillary leak syndrome due to C1 inhibitor deficiency complicating acute dermatomyositis: a case report"],"prefix":"10.1186","volume":"8","author":[{"given":"Ilse","family":"Gradwohl-Matis","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Romana","family":"Illig","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hermann","family":"Salmhofer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Daniel","family":"Neureiter","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andreas","family":"Brunauer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Martin W","family":"D\u00fcnser","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2014,1,27]]},"reference":[{"key":"2673_CR1","doi-asserted-by":"publisher","first-page":"C18","DOI":"10.12968\/hmed.2012.73.Sup2.C18","volume":"73","author":"S Mahil","year":"2012","unstructured":"Mahil S, Marks D, McCormack M, Rahman A: Dermatomyositis. Br J Hosp Med (Lond). 2012, 73: C18-C22.","journal-title":"Br J Hosp Med (Lond)"},{"key":"2673_CR2","doi-asserted-by":"publisher","first-page":"1851","DOI":"10.1007\/s10067-007-0588-z","volume":"26","author":"Y Sherer","year":"2007","unstructured":"Sherer Y, Shepshelovich D, Shalev T, Haviv Y, Segal E, Ehrenfeld M, Levy Y, Pauzner R, Shoenfeld Y, Langevitz P: Outcome of patients having dermatomyositis admitted to the intensive care unit. Clin Rheumatol. 2007, 26: 1851-1855. 10.1007\/s10067-007-0588-z.","journal-title":"Clin Rheumatol"},{"key":"2673_CR3","doi-asserted-by":"publisher","first-page":"898","DOI":"10.1007\/s10067-005-0053-9","volume":"25","author":"W de Castro GR","year":"2006","unstructured":"de Castro GR W, Appenzeller S, B\u00e9rtolo MB, Costallat LT: Acute dermatomyositis with subcutaneous generalized edema. Clin Rheumatol. 2006, 25: 898-900. 10.1007\/s10067-005-0053-9.","journal-title":"Clin Rheumatol"},{"key":"2673_CR4","doi-asserted-by":"publisher","first-page":"iv18","DOI":"10.1093\/rheumatology\/kel311","volume":"45","author":"IE Lundberg","year":"2006","unstructured":"Lundberg IE: The heart in dermatomyositis and polymyositis. Rheumatology. 2006, 45: iv18-iv21.","journal-title":"Rheumatology"},{"key":"2673_CR5","doi-asserted-by":"publisher","first-page":"686","DOI":"10.1002\/clc.21964","volume":"35","author":"L Zhang","year":"2012","unstructured":"Zhang L, Wang GC, Ma L, Zu N: Cardiac involvement in adult polymyositis or dermatomyositis: a systematic review. Clin Cardiol. 2012, 35: 686-691.","journal-title":"Clin Cardiol"},{"key":"2673_CR6","doi-asserted-by":"publisher","first-page":"997","DOI":"10.1016\/0002-8703(93)90720-T","volume":"126","author":"SH Yale","year":"1993","unstructured":"Yale SH, Adlakha A, Stanton MS: Dermatomyositis with pericardial tamponade and polymyositis with pericardial effusion. Am Heart J. 1993, 126: 997-999. 10.1016\/0002-8703(93)90720-T.","journal-title":"Am Heart J"},{"key":"2673_CR7","first-page":"464","volume":"149","author":"S Chraibi","year":"1998","unstructured":"Chraibi S, Ibnabdeljalil H, Habbal R, Bennis A, Tahiri A, Chraibi N: Pericardial tamponade as the first manifestation of dermatopolymyositis. Ann Med Interne (Paris). 1998, 149: 464-466.","journal-title":"Ann Med Interne (Paris)"},{"key":"2673_CR8","doi-asserted-by":"publisher","first-page":"386","DOI":"10.3109\/s10165-012-0661-6","volume":"23","author":"H Yamashita","year":"2012","unstructured":"Yamashita H, Matsuki Y, Shimizu A, Mochizuki M, Takahashi Y, Kano T, Mimori A: Hemophagocytic lymphohistiocytosis complicated by central nervous system lesions in a patient with dermatomyositis: a case presentation and literature review. Mod Rheumatol. 2012, 23: 386-392.","journal-title":"Mod Rheumatol"},{"key":"2673_CR9","doi-asserted-by":"publisher","first-page":"481","DOI":"10.1007\/s11739-010-0408-3","volume":"5","author":"M Cicardi","year":"2010","unstructured":"Cicardi M, Zanichelli A: Angioedema due to C1 inhibitor deficiency in 2010. Intern Emerg Med. 2010, 5: 481-486. 10.1007\/s11739-010-0408-3.","journal-title":"Intern Emerg Med"},{"key":"2673_CR10","doi-asserted-by":"publisher","first-page":"156","DOI":"10.1016\/j.autrev.2008.05.003","volume":"8","author":"M Cugno","year":"2008","unstructured":"Cugno M, Castelli R, Cicardi M: Angioedema due to acquired C1-inhibitor deficiency: a bridging condition between autoimmunity and lymphoproliferation. Autoimmun Rev. 2008, 8: 156-159. 10.1016\/j.autrev.2008.05.003.","journal-title":"Autoimmun Rev"},{"key":"2673_CR11","doi-asserted-by":"publisher","first-page":"2765","DOI":"10.1056\/NEJMcp052790","volume":"355","author":"J Blad\u00e9","year":"2006","unstructured":"Blad\u00e9 J: Clinical practice. Monoclonal gammopathy of undetermined significance. N Engl J Med. 2006, 355: 2765-2770. 10.1056\/NEJMcp052790.","journal-title":"N Engl J Med"},{"key":"2673_CR12","doi-asserted-by":"publisher","first-page":"563","DOI":"10.1136\/adc.87.6.563-a","volume":"87","author":"R Narasimhan","year":"2002","unstructured":"Narasimhan R, Lakshman R, Amos RS, Williams LH, Egner W, Finn A: Juvenile dermatomyositis associated with hereditary angioneurotic oedema. Arch Dis Child. 2002, 87: 563-10.1136\/adc.87.6.563.","journal-title":"Arch Dis Child"},{"key":"2673_CR13","doi-asserted-by":"publisher","first-page":"312","DOI":"10.2500\/aap.2013.34.3677","volume":"34","author":"K Bork","year":"2013","unstructured":"Bork K, Steffensen I, Machnig T: Treatment with C1-esterase inhibitor concentrate in type I or II hereditary angioedema: a systematic literature review. Allergy Asthma Proc. 2013, 34: 312-327. 10.2500\/aap.2013.34.3677.","journal-title":"Allergy Asthma Proc"},{"key":"2673_CR14","doi-asserted-by":"publisher","first-page":"597","DOI":"10.1016\/j.jaad.2005.10.041","volume":"54","author":"P Gerami","year":"2006","unstructured":"Gerami P, Schope JM, McDonald L, Walling HW, Sontheimer RD: A systematic review of adult-onset clinically amyopathic dermatomyositis (dermatomyositis sine myositis): a missing link within the spectrum of the idiopathic inflammatory myopathies. J Am Acad Dermatol. 2006, 54: 597-613. 10.1016\/j.jaad.2005.10.041.","journal-title":"J Am Acad Dermatol"},{"key":"2673_CR15","doi-asserted-by":"publisher","first-page":"639","DOI":"10.1097\/BOR.0b013e32833f1987","volume":"22","author":"S Sato","year":"2010","unstructured":"Sato S, Kuwana M: Clinically amyopathic dermatomyositis. Curr Opin Rheumatol. 2010, 22: 639-643. 10.1097\/BOR.0b013e32833f1987.","journal-title":"Curr Opin Rheumatol"}],"container-title":["Journal of Medical Case Reports"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/1752-1947-8-28.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1186\/1752-1947-8-28\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/1752-1947-8-28","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/1752-1947-8-28.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,9,2]],"date-time":"2021-09-02T02:56:48Z","timestamp":1630551408000},"score":1,"resource":{"primary":{"URL":"https:\/\/jmedicalcasereports.biomedcentral.com\/articles\/10.1186\/1752-1947-8-28"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2014,1,27]]},"references-count":15,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2014,12]]}},"alternative-id":["2673"],"URL":"https:\/\/doi.org\/10.1186\/1752-1947-8-28","relation":{},"ISSN":["1752-1947"],"issn-type":[{"value":"1752-1947","type":"electronic"}],"subject":[],"published":{"date-parts":[[2014,1,27]]},"assertion":[{"value":"20 August 2013","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"4 November 2013","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"27 January 2014","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}}],"article-number":"28"}}